EULAR study group on 'MHC-I-opathy': identifying disease-overarching mechanisms across disciplines and borders.

The 'MHC-I (major histocompatibility complex class I)-opathy' concept describes a family of inflammatory conditions with overlapping clinical manifestations and a strong genetic link to the MHC-I antigen presentation pathway. Classical MHC-I-opathies such as spondyloarthritis, Behçet's disease, psoriasis and birdshot uveitis are widely recognised for their strong association with certain MHC-I alleles and gene variants of the antigen processing aminopeptidases ERAP1 and ERAP2 that implicates altered MHC-I peptide presentation to CD8+T cells in the pathogenesis. Progress in understanding the cause and treatment of these disorders is hampered by patient phenotypic heterogeneity and lack of systematic investigation of the MHC-I pathway.Here, we discuss new insights into the biology of MHC-I-opathies that strongly advocate for disease-overarching and integrated molecular and clinical investigation to decipher underlying disease mechanisms. Because this requires transformative multidisciplinary collaboration, we introduce the EULAR study group on MHC-I-opathies to unite clinical expertise in rheumatology, dermatology and ophthalmology, with fundamental and translational researchers from multiple disciplines such as immunology, genomics and proteomics, alongside patient partners. We prioritise standardisation of disease phenotypes and scientific nomenclature and propose interdisciplinary genetic and translational studies to exploit emerging therapeutic strategies to understand MHC-I-mediated disease mechanisms. These collaborative efforts are required to address outstanding questions in the etiopathogenesis of MHC-I-opathies towards improving patient treatment and prognostication.

LENS CARE COMPLIANCE RATES AND PERCEPTIONS AMONG RIGID GAS PERMEABLE CONTACT LENS WEARERS - A PILOT STUDY.

The aim was to estimate compliance rate among rigid gas permeable lens wearers (RGPLW) in lens system care, identify procedures in lens care process with poorest compliance levels, and assess concordance between participant reported practices and their subjectively perceived compliance. The study included outpatient RGPLW managed at Zagreb University Hospital Center in Zagreb, Croatia. They filled out a questionnaire that included demographic data, duration of lens wear, self-evaluation compliance grade, and 14 lens care procedures and wearing habits indicative of compliance. There were 50 patients (mean age 34.6 years, 68% female). Full compliance was found in a single patient. The mean number of non-compliant procedures was 5.48, with 32% of participants non-compliant in more than 50% of the compliance criteria. Critical procedures of the lens care process were infrequent lens case exchange (74%), using tap water for lens (70%), and improper case cleaning (68%). The mean lens case replacement time was 9.8 months (SD 6.76), with only 26% of patients replacing lens case at least once in 3 months. Excessive daily lens wear was associated with greater total number of non-compliant procedures (p<0.0008). RGPLW were aware of their inappropriate lens care only when achieved non-compliance in almost 50% of the procedures. In conclusion, lens wearers were not aware of their extremely low compliance rate in several aspects of lens and lens case maintenance. Study results indicated the key procedures the practitioners should focus on when evaluating subjective and objective compliance and reinforcing care and hygiene education of RGPLW.

The Influence of Tear Film Quality on Visual Function.

BACKGROUND: The prevalence of dry eye disease (DED) is increasing globally, resulting in a variety of eye symptoms characterized by discomfort and visual disturbances. The accurate diagnosis of the disease is often challenging and complex, requiring specialized diagnostic tools. This study aimed to investigate the impact of tear film instability on visual function and to evaluate the value of post-blink blur time (PBBT) as an alternative method for assessing tear film stability. METHODS: The study included 62 subjects: 31 with subjective symptoms of DED (Group A) and a control group consisting of 31 healthy participants (Group B). Symptoms were assessed using the standard Schein questionnaire, supplemented with additional questions. PBBT was measured using standard Snellen charts to investigate a potential association between PBBT and tear film dysfunction. Additional clinical assessments included tear film break-up time (TBUT). RESULTS: Statistically significant differences were observed in the average values of PBBT and TBUT between the examined groups. The average PBBT was 8.95 ± 5.38 s in the group with DED and 14.66 ± 10.50 s in the control group, p < 0.001. Group A exhibited an average TBUT of 4.77 ± 2.37 s, while Group B had a TBUT of 7.63 ± 3.25 s, p < 0.001. Additionally, a strong positive correlation was identified between PBBT and TBUT values (r = 0.455; p < 0.001). CONCLUSIONS: The research confirms that tear film stability has an important role in the refraction of light and the maintenance of optical quality of vision. PBBT could potentially function as an objective and clinically significant screening test for DED.

Liver metastasis in uveal melanoma - treatment options and clinical outcome.

Uveal melanoma (UM) is the most prevalent primary intraocular malignancy in adults with a stable incidence rate between five and seven cases per million in Europe and the United States. Although UM and melanoma from other sites have the same origin, UM has different epidemiological, biological, pathological and clinical features including characteristic metastatic hepatotropism. Despite improvements in the treatment of primary tumours, approximately 50% of patients with UM will develop metastases. In 90% of cases the liver is the first site of metastasis, however the mechanisms underlying this hepatic tropism have not been elucidated. Metastatic disease is associated with a very poor prognosis with a median overall survival of 6 to 12 months. Currently, there is no standard systemic treatment available for metastatic UM and once liver metastases have developed, prognosis is relatively poor. In order to prolong survival, close follow-up in all patients with UM is recommended for early detection and treatment. The treatment of metastatic UM includes systemic chemotherapy, immunotherapy and molecular targeted therapy. Liver-directed therapies, such as resection, radioembolization, chemoembolization, immunoembolization, isolated and percutaneous liver perfusion as well as thermal ablation represent available treatment options. However, to date a consensus regarding the optimal method of treatment is still lacking and the importance of setting guidelines in the treatment and management of metastatic UM is becoming a priority. Improvement in knowledge and a better insight into tumour biology, immunology and metastatic mechanism may improve current treatment methods and lead to the development of new strategies paving the way for a personalized approach.

Bilateral juxtapapillary choroidal neovascularization secondary to Birdshot chorioretinopathy-case report.

Central vision loss, photopsia, floaters, and macular edema in a highly myopic patient can easily be misinterpreted as high myopia complications. In atypical cases, detailed examination and a thorough diagnostic workup are required to establish the proper diagnosis, which is often beyond the scope of diagnoses initially considered.